Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The sinonasal undifferentiated carcinoma (SNUC) is an aggressive and rare neoplasm arising in the nasal cavity and the paranasal sinuses. To date, over 50 cases of histologically proven SNUCs have been reported since its original description in 1986. Presenting symptoms include facial pain, nasal obstruction, diplopia, epistaxis, proptosis, and periorbital swelling. The histologic features of this neoplasm include cohesive cells arranged in nests, ribbons, and trabeculae. The cells exhibit hyperchromatic nuclei and a high nuclear to cytoplasmic ratio. A brisk mitotic rate, tumor necrosis, and vascular invasion are prominent features. Confirming the diagnosis of SNUC at the light microscopic level can be challenging, since the microscopic differential diagnosis includes olfactory neuroblastoma, rhabdomyosarcoma, undifferentiated nasopharyngeal carcinoma (lymphoepithelioma), malignant lymphoma, malignant melanoma, and neuroendocrine (small cell undifferentiated; oat cell) carcinoma. Sinonasal undifferentiated carcinoma can be differentiated from these other neoplasms by correlating clinical, light microscopic, histochemical, immunohistochemical, and ultrastructural characteristics. Aggressive, multimodal therapy can provide the best opportunity for local control of this neoplastic process, but the optimal treatment has yet to be determined.
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PMID:Sinonasal undifferentiated carcinoma: a distinctive clinicopathologic entity. 1056 93

Maffucci's syndrome consists of multiple cutaneous hemangiomas, dyschondroplasia, and enchondromas with potential for malignant change. We report a case of a 33-year-old man with Maffucci's syndrome who presented with a several month history of nasal congestion, facial pain, and diminished vision in his left eye. Radiological studies showed a large soft tissue mass centered in the sinonasal area, extending bilaterally into maxillary sinuses and orbits with compression of left optic nerve. Biopsy of the mass showed esthesioneuroblastoma (olfactory neuroblastoma). Chemotherapy resulted in initial improvement, but the tumor recurred and did not respond to further treatment, resulting in his death. Sarcomatous tumors are reported in Maffucci's syndrome, but this is a rare case of a neuroendocrine tumor in a patient with Maffucci's syndrome.
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PMID:Esthesioneuroblastoma in Maffucci's syndrome. 1522 Dec 18

Malignant tumors of the nasal cavity are rare. We report the case of an elderly woman who consulted us with a 4-year history of progressive nasal obstruction, occasional epistaxis, facial pain, and watering of the eyes. A diagnosis of olfactory neuroblastoma was established by histopathology and confirmed by immunohistochemistry. On staging, the mass was classified as a Kadish stage B tumor. The mass was excised via a lateral rhinotomy approach, and the tumor was peeled away completely from the cribriform plate with endoscopes. The patient underwent postoperative radiation, and she was free of recurrence at follow-up 15 months later.
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PMID:Olfactory neuroblastoma: a case report and review of the literature. 1587 82

Malignant tumors of the nasal cavity are rare. We report the case of a 48 years old man who consulted us with a 2-year history of progressive nasal obstruction, occasional epistaxis, facial pain, and watering of the eyes. A diagnosis of olfactory neuroblastoma was established by histopathology and confirmed by immunohistochemistry. On staging, the mass was classified as a Kadish stage C tumor with extensive involvement of the nasal cavities, nasopharynx, paranasal sinuses and orbit. Endoscopic excision of the mass was done. Traditionally the mainstay of treatment in such locally advanced patients is craniofacial resection followed by adjuvant radiotherapy. Our patient was treated with limited surgery and chemoradiation. Patient is free of recurrence at a follow-up of 5 years. This case report demonstrates the potential efficacy of planned combined modality therapy, including limited surgery and early chemoradiation in the control of locally advanced olfactory neuroblastoma.
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PMID:Esthesioneuroblastoma chemotherapy and radiotherapy for extensive disease: a case report. 2197 76

Esthesioneuroblastoma (ENB) also known as olfactory neuroblastoma is an uncommon malignant neoplasm arising in the roof of nasal cavity. It is now understood to originate from the olfactory epithelium. Case reports published worldwide have been very few. Common presenting symptoms of Esthesioneuroblastoma include nasal obstruction, epistaxis, facial pain, diplopia, proptosis, and anosmia. Apart from being locally aggressive, it metastasizes widely by both hematogenous and lymphatic routes.
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PMID:Esthesioneuroblastoma a case report. 2275 35

A 35-year-old female presented with 13-year history of unilateral recurrent nasal mass, epistaxis and facial pain. Nasal examination revealed a pale glistening mass in the right nasal cavity. On probing, mass was insensitive to touch and bled on handling. Computed tomographic scan showed a mass filling the right nasal cavity, ipsilateral maxillary and ethmoid sinuses. Diagnosis of pansinusitis polyposis was made.Transnasal endoscopy-assisted excision of the mass was done, and the diagnosis of olfactory neuroblastoma was established by histopathology and confirmed by immunohistochemistry. The mass was classified as a Kadish stage B tumor. Further intervention including medial maxillectomyand ethmoidectomy, and complete endoscopic-resection of the tumor from cribriform plate was done via lateral rhinotomy approach. The tumor was found adhered to the lateral wall-the middle meatus and was easily peeled away from the cribriform plate and ethmoids. Patient was referred for radiotherapy. No evidence of loco-regional recurrence or systemic metastasis observed at 10-month follow-up.
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PMID:Esthesioneuroblastoma arising from the middle meatus. 2357 84

Abstract Esthesioneuroblastoma, also known as olfactory neuroblastoma, is an uncommon malignant neoplasm arising from the olfactory epithelium in the roof of the nasal cavity. There are very few case reports published worldwide. The common presenting symptoms of Esthesioneuroblastoma are unilateral nasal obstruction (70%), epistaxis (50%), anosmia, rhinorrhoea, facial pain, headache, excessive lacrimation and rarely proptosis and visual disturbance. Apart from being locally aggressive, it metastasizes by haematogenous and lymphatic routes. We report an extremely rare case of esthesioneuroblastoma in a 20-year-old man with orbital involvement presenting as dystopia. This rare tumour should be considered in the differential diagnosis for young patients presenting to ophthalmic outpatient department with dystopia.
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PMID:Esthesioneuroblastoma as an unusual cause for dystopia. 2406 11

Esthesioneuroblastoma (ENB)-also known as olfactory neuroblastoma-originates primarily from the olfactory epithelium in the roof of the nasal cavity. We present here the 7th reported case of ENB arising from the sphenoidal sinus. We undertook a review of the case notes from the time of initial presentation and literature review of this topic. A 55-year-old male presented with a 3-year history of unilateral progressive nasal obstruction, epistaxis, difficult nasal-breathing, facial pain, frontal headache and blood-tinged discharge. Nasal examination revealed a glistening mass in right nasal cavity. Endoscopy-assisted transnasal excision was performed. Histologically, tumor was identified as small round cell tumor and confirmed by immunohistochemistry to be olfactory neuroblastoma (negative leucocyte common antigen, positive neurone specific enolase). Sphenoidal ENB is rare in the literature, and this presentation is the 7th reported case; in addition, surgical treatment is very challenging, and non-surgical treatment is used most commonly.
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PMID:Sphenoidal esthesioneuroblastoma treated with sequential chemo radiotherapy: a case report and review of the literature. 2557 62